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Successful treatment of three cases of nephrogenic fibrosing dermopathy with extracorporeal photopheresis
Author(s) -
Gilliet M.,
Cozzio A.,
Burg G.,
Nestle F.O.
Publication year - 2005
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.2005.06434.x
Subject(s) - medicine , extracorporeal photopheresis , photopheresis , contracture , dermatology , etiology , transplantation , pathological , surgery , dialysis , pathology , disease , graft versus host disease
Summary Background Nephrogenic fibrosing dermopathy (NFD) is a recently described cutaneous fibrosing disorder associated with renal dysfunction. Patients present with thickened skin or oedematous skin with indurated papules and plaques involving extremities and trunk, and often associated with disabling contracture of the adjacent joints. The aetiology and pathogenesis remain largely unknown. As a consequence, therapeutic measures with proven efficacy are nonexistent to date. Objectives To consider treatment with extracorporal photopheresis (ECP) in three patients. Patients We report three new cases of NFD with the characteristic clinical and pathological features. Two patients required haemodialysis due to end‐stage renal failure, despite prior renal transplantation. One patient had renal dysfunction but was never on dialysis, nor had she been transplanted. ECP treatment was administered at intervals of 2–4 weeks. Results All three patients showed a softening of the skin lesions and a marked improvement of the joint motility starting after four cycles of ECP. One patient developed a complete regression of her skin lesions after 16 cycles of ECP, and response to therapy was observed despite constantly elevated renal values. Conclusion These data indicate that ECP may represent a valuable therapeutic option for NFD.